CONGENITAL GALACTOSEMIA, A SINGLE ENZYMATIC BLOCK IN GALACTOSE METABOLISM
CONGENITAL GALACTOSEMIA, A SINGLE ENZYMATIC BLOCK IN GALACTOSE METABOLISM
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DOI：
10.1126/science.123.3198.635
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发表时间：
1956-01-01
期刊：
影响因子：
56.9
通讯作者：
KALCKAR, HM
中科院分区：
文献类型：
--
作者：
ISSELBACHER, KJ;ANDERSON, EP;KALCKAR, HM
68% 18% 10% 4% Vitamin suppl. ement (g/100 lb) Vitamin A conc.(200,000) 4.500 g Vitamin D conc.(400,000) 3.000 g Alpha-tocopherol 10.215 g Choline chloride 272.400 g Niacin 27.240 g Inocitol 13.620 g 2-Methyl-1, 4-naphthoquinone 0.1021 g Pyridoxine HCl 0.9534 g Riboflavin 0.9534 g Calcium pantothenate 2.0430 g aqueous solution of toluidine blue, and the other half was stained for lipids in a solution of sudan black B. A total of 95 biopsy specimens was studied. To insure that the mice would possess hair follicles at the proper stage of hair growth throughout the course of the experiments, club hairs were plucked from the resting follicles. Plucking of club hairs initiated a new wave of hair growth that required approximately 19 days for completion (2) in the plucked area only. The mice were placed on the thiamine-deficient diet at 21 days after plucking. The hairs at this time had just completed their growth and entered the resting phase and would remain in the resting phase for about 1 month (2). After 7 or 14 days of the deficiency regimen, one half of the dorsum of each mouse was again plucked, and in this replucked area hair growth was again initiated. Thus, throughout the remainder of the experiment, one half of the dorsum of each mouse had growving follicles and the other half had resting follicles.Mice that were fed the deficient diet tended to inerease in weight throughout the first week and level off during thesecond. About the 14th day, a progressive loss of weight began, and death occurred between the 21st and 25th days. Concomitant with the decrease in weight in the third week, the hair coat lost its smoothness, and the hairs felt dry to the touch. Although the pair-fed control animals lost much weight, their hair coat re-mained smooth and oily. Biopsy specimens of skin from both deficient and pair-fed control animals showed essentially a normal appearance 7 days after the initiation of the defi-ciency regimen.
